Lin et al. World Journal of Surgical Oncology (2015) 13:87 DOI 10.1186/s12957-015-0489-6
WORLD JOURNAL OF SURGICAL ONCOLOGY
RESEARCH
Open Access
Surgical experience of adult primary hepatic sarcomas Yu-Hung Lin1, Chih-Che Lin1, Allan M Concejero1, Chee-Chien Yong1, Fang-Ying Kuo2 and Chih-Chi Wang1*
Abstract Background: Primary hepatic sarcoma (PHS) is a rare primary liver malignancy. The histological types of PHS are diverse, and the clinical outcomes and management mainly depend on the histopathology. This study aims to evaluate the results of surgical intervention. Methods: Between January 2003 and June 2009, 13 adult patients with pathologically proven PHS were identified by record review. The patients’ demographic profile, tumor characteristics, treatment modalities, and outcomes were reviewed and analyzed. The end of follow-up was December 2014. Results: Nine (69%) underwent curative liver resection and two underwent liver transplantation; the others received non-operative treatments. The pathologic findings were six (46%) angiosarcomas, four (30.7%) undifferentiated sarcomas, one (7.6%) leiomyosarcoma, one (7.6%) malignant mesenchymoma, and one (7.6%) hepatic epithelioid hemangioendothelioma. The median follow-up was 31.4 (2.8 ~ 142.5) months. The 1-, 2-, and 5-year survival of surgical patients were 72.7%, 63.6%, and 36.4%, respectively. Importantly, the 1-, 2-, and 5-year survival rates of non-angiosarcoma patients were superior to those of angiosarcoma (85.7% vs. 33.3%, 71.4% vs. 16.7%, and 57.1% vs. 0%, respectively, P = 0.023). Conclusions: Surgical intervention provides the possibility of long-term survival from PHS. Angiosarcoma is associated with a more dismal outcome than non-angiosarcoma. Keywords: Primary hepatic sarcoma, Surgery, Angiosarcoma, Non-angiosarcoma, Outcome
Background Primary hepatic sarcoma (PHS) is a rare malignancy accounting for less than 1% of all liver malignancies [1-3]. The etiologies of this disease are still not well-known, unlike hepatocellular carcinoma (HCC) which is the most common histology of primary hepatic malignancy and closely associated with infection of viral hepatitis, alcoholism, and liver cirrhosis. The clinical presentations of PHS are usually non-specific such as abdominal pain and weight loss [4,5]. HCC and cholangiocarcinoma are associated with the elevation of alpha-fetoprotein (AFP) and carbohydrate antigen 19-9 (CA 19-9), respectively. But no tumor marker has been identified to accurately detect PHS. The treatment guidelines of HCC evolved and have been very well-established in recent decades * Correspondence: [email protected] 1 Department of Surgery of Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, No. 123, Da-Pei Road, Niao-Sung, Kaohsiung 83301, Taiwan Full list of author information is available at the end of the article
[4]. In contrast, PHS is less studied and no clinical guideline can be applied. PHS has a wide diversity of histological types. Leiomyosarcoma and angiosarcoma are the most common histological types [1,6-9]. Undifferentiated sarcoma most often occurs in childhood but is also common in adults. The histological nomenclature for undifferentiated sarcoma was not uniform before 1978. The names were mesenchymoma, primary sarcoma of liver, fibromyxosarcoma, and malignant mesenchymoma. The clinical outcomes and treatment are quite different in each type of histology. Radiotherapy is commonly used for extremity sarcoma, but its detrimental adverse effects when delivered at high doses to abdominal organs limit its application when treating hepatic sarcoma [1]. Without surgery, chemotherapy provides minimal benefits of survival in sarcomas. In the past two decades, substantial improvement in surgical technique, perioperative management, and earlier diagnosis have resulted in
© 2015 Lin et al.; licensee BioMed Central. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Lin et al. World Journal of Surgical Oncology (2015) 13:87
marked reduction in operative and hospital mortality rates for patients undergoing hepatectomy and liver transplantation (LT) [10-13]. Aggressive surgical approach seems to be the only effective treatment to achieve possible long-term survival for PHS [7]. Since PHS is rare, the course of disease, prognostic factors, and consensus on management are not wellclarified. The aim of this study is to analyze our surgical experience in the management of this rare malignancy.
Methods We retrospectively reviewed the records of 13 adult patients with PHS confirmed by pathology at Kaohsiung Chang Gung Memorial Hospital from January 2003 to June 2009. Patients were followed up till December 2014. This study was approved by the Institutional Review Board of this hospital. Written informed consent was obtained from the patient for the publication of this report and any accompanying images. The diagnostic imaging modalities were abdominal ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) with or without hepatic angiography. Laboratory blood tests included hepatitis B antigen and antibody, hepatitis C antibody, serum AFP, carcinoembryonic antigen (CEA), CA 19-9, serum albumin, serum total bilirubin, aspartate aminotransferase, alanine aminotransferase, differentiated blood cell counts, international normalized ratio, and prothrombin time. Patients who underwent work-up for possible LT underwent other examinations that included panendoscopy, colonoscopy, chest and brain images, and bone scan to screen out possible distant metastases or double cancers. Patients who were identified as candidates for hepatic resection underwent indocyanine green clearance test [10,11]. Resectability was determined by tumor extent, possible residual liver volume on imaging, and biochemical tests especially indocyanine green clearance test [14]. Patients with resectable liver tumors and had resectable solitary lung metastasis were also considered as resection candidates. The surgery types were hepatectomy (anatomical and non-anatomical) and LT (brain-death deceased donor and living-related donor). The procedures followed standardized surgical protocols and techniques and were described elsewhere in detail [15-17]. The resection margin was defined as follows: R0: no residual tumor; R1: microscopic residual tumor; and R2: macroscopic residual tumor [18]. The patients were followed in the out-patient clinic at 1 month after the operative procedure and every 3 months thereafter with regular abdominal ultrasound, liver function test, and tumor markers. If there was a suspicious recurrence, computed tomography scan or magnetic resonance imaging was performed.
Page 2 of 6
For patients with unresectable or recurrent disease, transarterial embolization, radio frequency ablation (RFA), percutaneous ethanol injection (PEI), chemotherapy, or combined treatments were applied. The descriptive statistics were presented with median (range). The overall survival, disease-free survival, and survival between different histological types were determined by the Kaplan-Meier method and compared by the log-rank test. A P value
WORLD JOURNAL OF SURGICAL ONCOLOGY
RESEARCH
Open Access
Surgical experience of adult primary hepatic sarcomas Yu-Hung Lin1, Chih-Che Lin1, Allan M Concejero1, Chee-Chien Yong1, Fang-Ying Kuo2 and Chih-Chi Wang1*
Abstract Background: Primary hepatic sarcoma (PHS) is a rare primary liver malignancy. The histological types of PHS are diverse, and the clinical outcomes and management mainly depend on the histopathology. This study aims to evaluate the results of surgical intervention. Methods: Between January 2003 and June 2009, 13 adult patients with pathologically proven PHS were identified by record review. The patients’ demographic profile, tumor characteristics, treatment modalities, and outcomes were reviewed and analyzed. The end of follow-up was December 2014. Results: Nine (69%) underwent curative liver resection and two underwent liver transplantation; the others received non-operative treatments. The pathologic findings were six (46%) angiosarcomas, four (30.7%) undifferentiated sarcomas, one (7.6%) leiomyosarcoma, one (7.6%) malignant mesenchymoma, and one (7.6%) hepatic epithelioid hemangioendothelioma. The median follow-up was 31.4 (2.8 ~ 142.5) months. The 1-, 2-, and 5-year survival of surgical patients were 72.7%, 63.6%, and 36.4%, respectively. Importantly, the 1-, 2-, and 5-year survival rates of non-angiosarcoma patients were superior to those of angiosarcoma (85.7% vs. 33.3%, 71.4% vs. 16.7%, and 57.1% vs. 0%, respectively, P = 0.023). Conclusions: Surgical intervention provides the possibility of long-term survival from PHS. Angiosarcoma is associated with a more dismal outcome than non-angiosarcoma. Keywords: Primary hepatic sarcoma, Surgery, Angiosarcoma, Non-angiosarcoma, Outcome
Background Primary hepatic sarcoma (PHS) is a rare malignancy accounting for less than 1% of all liver malignancies [1-3]. The etiologies of this disease are still not well-known, unlike hepatocellular carcinoma (HCC) which is the most common histology of primary hepatic malignancy and closely associated with infection of viral hepatitis, alcoholism, and liver cirrhosis. The clinical presentations of PHS are usually non-specific such as abdominal pain and weight loss [4,5]. HCC and cholangiocarcinoma are associated with the elevation of alpha-fetoprotein (AFP) and carbohydrate antigen 19-9 (CA 19-9), respectively. But no tumor marker has been identified to accurately detect PHS. The treatment guidelines of HCC evolved and have been very well-established in recent decades * Correspondence: [email protected] 1 Department of Surgery of Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, No. 123, Da-Pei Road, Niao-Sung, Kaohsiung 83301, Taiwan Full list of author information is available at the end of the article
[4]. In contrast, PHS is less studied and no clinical guideline can be applied. PHS has a wide diversity of histological types. Leiomyosarcoma and angiosarcoma are the most common histological types [1,6-9]. Undifferentiated sarcoma most often occurs in childhood but is also common in adults. The histological nomenclature for undifferentiated sarcoma was not uniform before 1978. The names were mesenchymoma, primary sarcoma of liver, fibromyxosarcoma, and malignant mesenchymoma. The clinical outcomes and treatment are quite different in each type of histology. Radiotherapy is commonly used for extremity sarcoma, but its detrimental adverse effects when delivered at high doses to abdominal organs limit its application when treating hepatic sarcoma [1]. Without surgery, chemotherapy provides minimal benefits of survival in sarcomas. In the past two decades, substantial improvement in surgical technique, perioperative management, and earlier diagnosis have resulted in
© 2015 Lin et al.; licensee BioMed Central. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Lin et al. World Journal of Surgical Oncology (2015) 13:87
marked reduction in operative and hospital mortality rates for patients undergoing hepatectomy and liver transplantation (LT) [10-13]. Aggressive surgical approach seems to be the only effective treatment to achieve possible long-term survival for PHS [7]. Since PHS is rare, the course of disease, prognostic factors, and consensus on management are not wellclarified. The aim of this study is to analyze our surgical experience in the management of this rare malignancy.
Methods We retrospectively reviewed the records of 13 adult patients with PHS confirmed by pathology at Kaohsiung Chang Gung Memorial Hospital from January 2003 to June 2009. Patients were followed up till December 2014. This study was approved by the Institutional Review Board of this hospital. Written informed consent was obtained from the patient for the publication of this report and any accompanying images. The diagnostic imaging modalities were abdominal ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) with or without hepatic angiography. Laboratory blood tests included hepatitis B antigen and antibody, hepatitis C antibody, serum AFP, carcinoembryonic antigen (CEA), CA 19-9, serum albumin, serum total bilirubin, aspartate aminotransferase, alanine aminotransferase, differentiated blood cell counts, international normalized ratio, and prothrombin time. Patients who underwent work-up for possible LT underwent other examinations that included panendoscopy, colonoscopy, chest and brain images, and bone scan to screen out possible distant metastases or double cancers. Patients who were identified as candidates for hepatic resection underwent indocyanine green clearance test [10,11]. Resectability was determined by tumor extent, possible residual liver volume on imaging, and biochemical tests especially indocyanine green clearance test [14]. Patients with resectable liver tumors and had resectable solitary lung metastasis were also considered as resection candidates. The surgery types were hepatectomy (anatomical and non-anatomical) and LT (brain-death deceased donor and living-related donor). The procedures followed standardized surgical protocols and techniques and were described elsewhere in detail [15-17]. The resection margin was defined as follows: R0: no residual tumor; R1: microscopic residual tumor; and R2: macroscopic residual tumor [18]. The patients were followed in the out-patient clinic at 1 month after the operative procedure and every 3 months thereafter with regular abdominal ultrasound, liver function test, and tumor markers. If there was a suspicious recurrence, computed tomography scan or magnetic resonance imaging was performed.
Page 2 of 6
For patients with unresectable or recurrent disease, transarterial embolization, radio frequency ablation (RFA), percutaneous ethanol injection (PEI), chemotherapy, or combined treatments were applied. The descriptive statistics were presented with median (range). The overall survival, disease-free survival, and survival between different histological types were determined by the Kaplan-Meier method and compared by the log-rank test. A P value
